Proteins/UNIPROT:Q92834

RPGR

ProteinUNIPROT:Q92834

Acts as a guanine-nucleotide releasing factor (GEF) for RAB8A and RAB37 by promoting the conversion of inactive RAB-GDP to the active form RAB-GTP. GEF activity towards RAB8A may facilitate ciliary trafficking by modulating ciliary intracellular localization of RAB8A. GEF activity towards RAB37 maintains autophagic homeostasis and retinal function (By similarity). Involved in photoreceptor integrity (By similarity). May control cilia formation by regulating actin stress filaments and cell contractility. May be involved in microtubule organization and regulation of transport in primary cilia. May play a critical role in spermatogenesis and in intraflagellar transport processes (By similarity)..

Function from UniProtKB (Q92834), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

5 rows

cone-rod dystrophy has a germline basis in RPGRDiseaseGenCC HGNC:10295|MONDO:0015993Inferred only
primary ciliary dyskinesia has a germline basis in RPGRDiseaseGenCC HGNC:10295|MONDO:0016575Inferred only
retinitis pigmentosa has a germline basis in RPGRDiseaseGenCC HGNC:10295|MONDO:0019200Inferred only
retinitis pigmentosa 3 has a germline basis in RPGRDiseaseGenCC HGNC:10295|MONDO:0010227Inferred only
RPGR-related retinopathy has a germline basis in RPGRDiseaseGenCC HGNC:10295|MONDO:0100437Inferred only

Statements

15 statements

15 statements · 3 reference databases · Subject: RPGR · Sources: gene ontology, Hetionet, WikiPathways

Order

DCTN1 interacts with RPGRProteinHetionet hetionet:interacts:1639-6103Inferred only
RPGR interacts with DCTN2ProteinHetionet hetionet:interacts:10540-6103Inferred only
RPGR interacts with IFT88ProteinHetionet hetionet:interacts:6103-8100Inferred only
RPGR interacts with KIF3AProteinHetionet hetionet:interacts:11127-6103Inferred only
RPGR interacts with KIFAP3ProteinHetionet hetionet:interacts:22920-6103Inferred only
NPM1 interacts with RPGRProteinHetionet hetionet:interacts:4869-6103Inferred only
PDE6D interacts with RPGRProteinHetionet hetionet:interacts:5147-6103Inferred only
RPGR interacts with RPGRIP1ProteinHetionet hetionet:interacts:57096-6103Inferred only
SLC25A10 interacts with RPGRProteinHetionet hetionet:interacts:1468-6103Inferred only
RPGR interacts with SMC1AProteinHetionet hetionet:interacts:6103-8243Inferred only
RPGR interacts with SMC3ProteinHetionet hetionet:interacts:6103-9126Inferred only
RPGR interacts with TUBG1ProteinHetionet hetionet:interacts:6103-7283Inferred only

Show the remaining 3

RPGR interacts with YWHAEProteinHetionet hetionet:interacts:6103-7531Inferred only
RPGR participates in Ciliary landscapePathwayWikiPathways WP4352Inferred only
RPGR regulates positive regulation of autophagyBiological processgene ontology Q92834|GO:0010508Inferred only

Other names

3 names

Resolves to: RPGR

Also called: RP3X-linked retinitis pigmentosa GTPase regulatorXLRP3

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